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Names & Taxonomy

Uniprot ID:: P01009
Entry Name:: A1AT_HUMAN
Status:: reviewed
Protein Names:: Alpha-1-antitrypsin (Alpha-1 protease inhibitor) (Alpha-1-antiproteinase) (Serpin A1) [Cleaved into: Short peptide from AAT (SPAAT)]
Gene Names:: SERPINA1 AAT PI PRO0684 PRO2209
Gene Names Orf:: PRO0684 PRO2209
Gene Names Primary:: SERPINA1
Organism:: Homo sapiens (Human)

Structure

Length:: 418
Sequence:: MPSSVSWGILLLAGLCCLVPVSLAEDPQGDAAQKTDTSHHDQDHPTFNKITPNLAEFAFSLYRQLAHQSNSTNIFFSPVSIATAFAMLSLGTKADTHDEILEGLNFNLTEIPEAQIHEGFQELLRTLNQPDSQLQLTTGNGLFLSEGLKLVDKFLEDVKKLYHSEAFTVNFGDTEEAKKQINDYVEKGTQGKIVDLVKELDRDTVFALVNYIFFKGKWERPFEVKDTEEEDFHVDQVTTVKVPMMKRLGMFNIQHCKKLSSWVLLMKYLGNATAIFFLPDEGKLQHLENELTHDIITKFLENEDRRSASLHLPKLSITGTYDLKSVLGQLGITKVFSNGADLSGVTEEAPLKLSKAVHKAVLTIDEKGTEAAGAMFLEAIPMSIPPEVKFNKPFVFLMIEQNTKSPLFMGKVVNPTQK
Proteomes:: UP000005640

Subcellular location

Subcellular Location:: Secreted. Endoplasmic reticulum. Note=The S and Z allele are not secreted effectively and accumulate intracellularly in the endoplasmic reticulum.; Short peptide from AAT: Secreted, extracellular space, extracellular matrix.

Function

Function:: Inhibitor of serine proteases. Its primary target is elastase, but it also has a moderate affinity for plasmin and thrombin. Irreversibly inhibits trypsin, chymotrypsin and plasminogen activator. The aberrant form inhibits insulin-induced NO synthesis in platelets, decreases coagulation time and has proteolytic activity against insulin and plasmin.; Short peptide from AAT: reversible chymotrypsin inhibitor. It also inhibits elastase, but not trypsin. Its major physiological function is the protection of the lower respiratory tract against proteolytic destruction by human leukocyte elastase (HLE).
Gene Ontology Go:: endoplasmic reticulum
endoplasmic reticulum lumen
endoplasmic reticulum-Golgi intermediate compartment membrane
ER to Golgi transport vesicle
extracellular exosome
extracellular region
extracellular space
Golgi apparatus
Golgi membrane
platelet alpha granule lumen
proteinaceous extracellular matrix
glycoprotein binding
identical protein binding
protease binding
serine-type endopeptidase inhibitor activity
acute-phase response
blood coagulation
cellular protein metabolic process
COPII vesicle coating
ER to Golgi vesicle-mediated transport
membrane organization
negative regulation of endopeptidase activity
platelet activation
platelet degranulation
post-translational protein modification
protein N-linked glycosylation via asparagine
Gene Ontology Biological Process:: acute-phase response
blood coagulation
cellular protein metabolic process
COPII vesicle coating
ER to Golgi vesicle-mediated transport
membrane organization
negative regulation of endopeptidase activity
platelet activation
platelet degranulation
post-translational protein modification
protein N-linked glycosylation via asparagine
Gene Ontology Molecular Function:: glycoprotein binding
identical protein binding
protease binding
serine-type endopeptidase inhibitor activity
Gene Ontology Cellular Component:: endoplasmic reticulum
endoplasmic reticulum-Golgi intermediate compartment membrane
endoplasmic reticulum lumen
ER to Golgi transport vesicle
extracellular exosome
extracellular region
extracellular space
Golgi apparatus
Golgi membrane
platelet alpha granule lumen
proteinaceous extracellular matrix
Keywords:: 3D-structure
Acute phase
Alternative splicing
Blood coagulation
Complete proteome
Direct protein sequencing
Endoplasmic reticulum
Extracellular matrix
Glycoprotein
Hemostasis
Phosphoprotein
Polymorphism
Protease inhibitor
Reference proteome
Secreted
Serine protease inhibitor
Signal
Interacts With:: Itself; P00760; P00772; P71213; P43307

Names & Taxonomy

Structure

Subcellular location

Function

Publication